An Overview of Idiopathic Pulmonary Fibrosis

Dr.Shaney L. Barratt at Bristol Interstitial Lung Disease Service, North Bristol NHS Trust, UK and colleagues have very beautifully presented an Overview of Idiopathic Pulmonary Fibrosis that has appeared in the Journal of Clinical Medicine.

The interstitial lung diseases (ILDs) are a heterogeneous group of parenchymal lung diseases characterised by varying degrees of inflammation and fibrosis. At times it may have secondary causes like drugs, autoimmune connective tissue disease, hypersensitivity to inhaled organic antigens, or sarcoidosis, whilst others, the idiopathic interstitial pneumonia (IIPs), have no identifiable cause.

• IPF is the most common form of ILD, interstitial lung disease.


• Globally, the incidence of the disease is rising, with associated high morbidity, mortality, and economic healthcare burden.


• Mortality in Idiopathic Pulmonary Fibrosis,IPF is high, with a reported median survival of 2–3 years from diagnosis, based on historical data


• International guidelines recommend that the diagnosis of Idiopathic Pulmonary Fibrosis,IPF be made at a multi-disciplinary level, requiring the exclusion of known causes of ILD and the presence of a usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) or surgical lung biopsy.


• In case there is an absence of honeycomb cysts but all other findings strongly indicate the presence of disease, guidelines suggested confirmation be sought through histological examination of surgical biopsy specimens.


• In clinical practice, surgical biopsy rates are low due to concerns regarding associated morbidity and mortality, particularly in an elderly co-morbid patient group


• A radiological diagnosis of UIP can be made based on HRCT alone in the presence of honeycomb cysts and subpleural, basally predominant reticulation, with or without traction bronchiectasis/bronchiectasis, negating the need for surgical biopsy.


• The treatment approach to Idiopathic Pulmonary Fibrosis,IPF has evolved considerably over the last two decades.


• Over recent years, two novel antifibrotic therapies, pirfenidone and nintedanib, have been developed, providing treatment options for many patients with IPF.


• Both antifibrotic agents have similar efficacy. Treatment decisions are generally driven by tolerance to side effect profiles.


• Current efforts are directed at identifying key biomarkers that may direct more customized patient-centred healthcare to improve outcomes for these patients in the future.

In spite of lot of progress made over recent years to improve our understanding of this condition and provide therapeutic options, there is still much work to be done to identify key targets that may allow preventative interventions and to develop strategies and/or biomarkers to aid early diagnosis or even to suspend fibrogenesis in this devastating condition.

doi:10.3390/jcm7080201